fibromuscular dysplasia life expectancy

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▷ What is the life expectancy of someone with Fibromuscular dysplasia?No resultsNo resultsProcessing results.... What is the prognosis of fibromuscular dysplasia (FMD)? See the following list: There are no statistics on the natural course of the stroke, especially since most cases are symptomatic and relatively benign. The series of cases tends to present a relatively favorable picture of long-term untraceed survival, both in medically and surgically administered patients. For example, in a 1981 report, Collins et al monitored 18 patients after surgical dilation for a half of just over 4 years, and none had strokes. [] They supervised 5 patients with global symptoms (e.g., hypoperfusion and non-hospital events) and conservative therapy for an average of 42 months, and none had strokes. The rupture of the sacral aneurysm has such a high mortality that the autopsy series can be biased in its FMD detection. The presence of saccular aneurysms probably poses the greatest threat of morbidity and mortality, especially if blood pressure is not controlled. In a prospective series of patients with carotid artery dissection observed for an average of 4 years, of those with recurrence (5 of 103), 80% had FMD. [] Related questions:Luscher TF, Lie JT, Stanson AW, et al. Arterial fibromuscular dysplasia. May Clin Proc. 1987 Oct. 62(10):931-52. Gray GH, Young JR, Olin JW. Miscellaneous arterial diseases. Young JR, Olin JW, Bartolomé J, Eds. Peripheral vascular diseases. 2a ed. St Louis: Mosby-Yearbook; 1996. 425-40. James TN. Morphological characteristics and functional meaning of the focal fibromuscular dysplasia of the small coronary arteries. Am J Cardiol. 1990 Apr 3. 65(14):12G-22G. . Campman SC, Holmes JF, Sokolove PE, et al. Pulmonary arterial fibromuscular dysplasia: a rare cause of fulminant pulmonary bleeding. Am J Forensic Med Pathol. 2000 Mar. 21(1):69-73. Maresi E, Becchina G, Ottoveggio G, et al. Arrhythmic sudden cardiac death in a 3-year-old child with intimal fibroplasia of coronary arteries, aorta and its branches. Cardiovasc Pathol. 2001 Jan-Feb. 10(1):43-8. . Luscher TF, Keller HM, Imhof HG, et al. Fibromuscular hyperplasia: extension of the disease and therapeutic result. Results of the University Hospital Zurich Cooperative Study on Fibromuscular Hyperplasia. Nephron. 1986. 44 Suppl 1:109-14. Hill LD, Antonius JI. Arterial dysplasia: a major surgical lesion. Arch Surg. 1965 Apr 90:585-95. Heffelfinger MJ, Holley KE, Havrison EG. Arterial fibromuscular Displasia studied in the autopsy [abstract]. Am J Clin Pathol. 1970. 54:274. Schievink WI, Bjornsson J. Fibromuscular dysplasia of the internal carotid artery: a clinicalopathological study. Clin Neuropathol. 1996 Jan-Feb. 15(1):2-6. Harrison EG Jr, McCormack LJ. Pathological classification of renal arterial disease in renovascular hypertension. May Clin Proc. 1971 Mar. 46(3):161-7. Stanley JC, Gewertz BL, Bove EL, et al. arterial fibrodysplasia. Histopathological character and current etiological concepts. Arch Surg. 1975 May. 110(5):561-6. Begelman SM, Olin JW. Fibromuscular dysplasia. Curr Opin Rheumatol. 2000 Jan. 12(1):41-7. . Reiher L, Pfeiffer T, Sandmann W. Long-term results after surgical reconstruction for fibromuscular dysplasia of renal artery. Eur J Vasc Endovasc Surg. 2000 Dec. 20(6):556-9. Mettinger KL, Ericson K. Fibromuscular dysplasia and brain. I. Observations on angiographic, clinical and genetic characteristics. Stroke. 1982 Jan-Feb. 13(1):46-52. . Cloft HJ, Kallmes DF, Kallmes MH, et al. Prevalence of cerebral aneurysms in patients with fibromuscular dysplasia: revaluation. J Neurosurg. 1998 Mar. 88(3):436-40. . Arunodaya GR, Vani S, Shankar SK, et al. Fibromuscular Displasia with Basilar artery dissection presenting as "blocking in syndrome". Neurology. 1997 Jun. 48(6):1605-8. . Cadaempati SR, Sebastian MW, Reed RL 2nd. Post-traumatic bilateral carotid artery and right vertebral arteries in a patient with fibromuscular dysplasia: case report and literature review. J Trauma. 1998 Feb. 44(2):406-9. Rushton AR. Genetics of fibromuscular dysplasia. Arch Intern Med. 1980 Feb. 140(2):233-6. . Bigazzi R, Bianchi S, Quilici N, et al. Bilateral fibromuscular displasia in identical twins. Am J Kidney Dis. 1998 Dec. 32(6):E4. . Tromp G, Wu Y, Prockop DJ, et al. The cDNA sequence of 50 non-related patients reveals that mutations in the triple-helical domain of type III procollagen are a rare cause of aortic aneurysms. J Clin Invest. 1993 Jun. 91(6):2539-45. McKusick VA. Heritable disorders of connective tissue. 4th edition. St Louis: Mosby-Yearbook; 1972. 382-6. Schievink WI, Meyer FB, Parisi JE, Wijdicks EF. Fibromuscular dysplasia of the internal carotid artery associated with alpha1-antitripsin deficiency. Neurosurgery. 1998 Aug. 43(2):229-33; discussion 233-4. Bray JM, Marc G, Pautot V, Vielle B, Pasco A, Lhoste P. Fibromuscular dysplasia can herald the symptomatic recurrence of cervical artery dissection. Cerebrovasc Dis. 2007. 23(5-6):448-52. Lee EK, Hecht ST, Lie JT. Multiple intracranial and systemic aneurysms associated with infant-joint arterial fibromuscular dysplasia. Neurology. 1998 Mar. 50(3):828-9. Kim SD, Park JO, Kim SH, Lee YH, Lim DJ, Park JY. Spontaneous spinal spontaneous subdural hematoma associated with fibromuscular dysplasia. J Neurosurg Spine. 2008 May. 8(5):478-81. Mettinger KL. Fibromuscular dysplasia and brain. II. Current concept of disease. Stroke. 1982 Jan-Feb. 13(1):53-8. . de Monyé C, Dippel DW, Dijkshoorn ML, Tanghe HL, van der Lugt A. Detection of MDCT of the fibromuscular dysplasia of the internal carotid artery. AJR Am J Roentgenol. April 2007. 188(4):W367-9. . Sabharwal R, Vladica P, Coleman P. Multidetector renal angiography in spiral in diagnosis of fibromuscular dysplasia of renal artery. Eur J Radiol. March 2007. 61(3):520-7. . Willoteaux S, Faivre-Pierret M, Moranne O, Lions C, Bruzzi J, Finot M, et al. Fibromuscular dysplasia of the main renal arteries: comparison of contrasted angiography with digital substraction angiography. Radiology. December 2006. 241(3):922-9. Bragin MA, Cherkasov AP. [Morphogenesis of fibromuscular dysplasia of renal arteries (anultrastructural study)]. Arkh Patol. 1979. 41(2):46-52. Kimura H, Hosoda K, Hara Y, Kohmura E. A very unusual case of fibromuscular dysplasia with multiple aneurysms of vertebral artery and posterior lower cerebellar artery. J Neurosurg. 2008 Dec. 109(6):1108-12. Van Damme H, Sakalihasan N, Limet R. Fibromuscular Displasia of the internal carotid artery. Personal experience with 13 cases and literary review. Acta Chir Belg. 1999 Aug. 99(4):163-8. . Chiche L, Bahnini A, Koskas F, Kieffer E. Occlusive fibromuscular disease of the arteries that supply the brain: results of surgical treatment. Ann Vasc Surg. 1997 Sep. 11(5):496-504. . Collins GJ Jr, Rich NM, Clagett GP, et al. Fibromuscular dysplasia of internal carotid arteries. Clinical experience and follow-up. Ann Surg. 1981 Jul. 194(1):89-96. . Finsterer J, Strassegger J, Haymerle A, Hagmüller G. Bilateral stenosis of the symptomatic and asymptomatic internal carotid artery due to fibromuscular dysplasia. Psychiatry of Neurosurgery J. 2000 Nov. 69(5):683-6. Leadbetter WF, Burkland CD. Hypertension in unilateral kidney disease. J Urol. 1938. 39:611-26. MC Leary, Finley A, Caplan LR. Complications Cerebrovasculars of fibromuscular dysplasia. Curr Treat Options Cardiovasc Med. 2004 Jun. 6(3):237-248. Olin JW. Recognizing and managing fibromuscular dysplasia. Cleve Clin J Med. 2007 Abr. 74(4):273-4, 277-82. . Palubinskas AJ, Ripley HR. Fibromuscular hyperplasia in extrarenal arteries. Radiology. 1946. 82:451-55. Saw J, Ricci D, Starovoytov A, Fox R, Buller CE. Spontaneous coronary dissection: prevalence of predisposing conditions including fibromuscular dysplasia in a tertiary central cohort. JACC Cardiovasc Interv. 2013 Jan 6(1):44-52. .Vuong PN, Desoutter P, Mickley V. Fibromuscular dysplasia of the renal artery responsible for renovascular hypertension: a histological presentation based on a series of 102 patients. Vasa. 2004 Feb. 33(1):13-8. Wood S. More evidence linking spontaneous coronary dissection and FMD. Medscape Medical News. 9 January 2013. .James A Wilson, MD, MSc, FRCPC Neurologist and Clinical Neurophysiologist, Oconee Neurology Services James A Wilson, MD, MSc, FRCPC is a member of the following medical societies: , Disclosure: Nothing to reveal. Richard L Hughes, Professor of Neurology at the University of Colorado at the Faculty of Medicine in Denver; Head of the Neurology Division, Denver Medical Center Richard L Hughes, MD is a member of the following medical societies: , , , Disclosure: Nothing to reveal. Francisco Talavera, PharmD, PhD Associate Professor, University of Nebraska Medical Center College of Pharmacy; Chief Editor, Medscape Drug ReferenceDisclosure: Received Medscape's salary for employment. Medscape. Howard S Kirshner, MD Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice President of the Department of Neurology, School of Medicine of the Vanderbilt University; Director of the Vanderbilt Stroke Center; Director of the Program, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Cabinet, Department of Neurology, Medical Center for Veterans Affairs of Nashville Howard S Kirshner, MD is a member of the following medical societies: , , , , , , , , , Disclosure: Nothing to reveal. Helmi L Lutsep, MD Professor and Vice Chair, Department of Neurology, Oregon Health and Science University School of Medicine; Associate Director, OHSU Stroke Center Helmi L Lutsep, MD is a member of the following medical societies: , Disclosure: Medscape Neurology Editorial Board for: Stroke Adjudication Committee, CREST2; Medical Advisory Board for Coherex Medical; National Leader and Board of Directors in Clinical Test, Bristol Myers Squibb; Consultant, Abbott Vascular, Inc. Jeffrey L Saver, U.S. Medical Professor. Jeffrey L Saver, MD, FAHA, FAAN is a member of the following medical societies: , , , Disclosure: Received the University of California Regents receives funding for consultancy services in the design of clinical trials provided to covidien, stryker and lundbeck. University of California to consult. Find us aboutMembershipWebMD NetworkEditions

Rare Diseases DatabaseFrommuscular DisplasiaNORD acknowledges with appreciation Pamela Mace, RN, Executive Director of the American Fibromuscular Society of Displasia and Heather Gornik, MD, Medical Director, Non-Invasive Vascular Laboratory, Cleveland Clinic Heart and Vascular Institute, to receive assistance in preparing this report. Synonyms of fibromuscular dysplasia General DiscussionThe fibromuscular dysplasia, commonly called FMD, is a disease that causes one or more arteries of the body to have abnormal cell development on the wall of the artery. As a result, narrowing areas (stenosis), aneurysms or tears (dissection) may occur. If narrowing or tear causes a decrease in blood flow through the artery, symptoms may result. The FMD is most commonly found in the arteries that supply the kidneys with blood (renal arteries) and the arteries called the carotid and vertebral arteries found in the neck and supply blood to the brain. Less commonly, FMD affects the arteries of the abdomen (supplying the liver, spleen and intestines) and extremities (legs and arms). In more than half of people with this disease, there will be evidence of FMD in more than one artery. Recently, some studies have shown that some women who were healthy and who have a sudden tear from a coronary artery (called sudden coronary dissection or "SCAD") probably have an undiagnosed FMD. SCAD was previously thought as a separate condition for the FMD, but we are now beginning to understand that many people have the FMD as the main reason for their coronary tear. Signs " Symptoms Some people with this disease have no symptoms or findings in a physical examination. Signs and/or symptoms that a person with FMD may experience depend on the affected arteries and if there is closeness, tears or aneurysms within them. Any pain or clinical sign related to MDF usually comes from the organ that is supplied by that artery. For example, FMD in renal arteries can cause high blood pressure. The FMD in carotid arteries can cause headaches or a sound in the ears (called pulsatile tinnitus). Some DFF patients may not have symptoms, but are diagnosed with this disease when a doctor hears a noise about one of the arteries due to disturbed or turbulent blood flow within the vessel. This noise is known as a brute. A person with a severe carotidic FMD that causes severe narrowing or tear in a carotid or vertebral artery may have neurological symptoms involving facial nerves (drooping of the eye cap, unequal size of the pupils, for example), ischemic stroke or transit. People with FMD carotide have a higher risk of aneurysms of the arteries in the brain (intrarracan aneurysms). Bleeding in the brain (intrarrhea hemorrhage) can occur if a break of aneurysm, and it is important to identify and treat brain aneurysms early to prevent this. The FMD that involves the arteries that supply the intestines, liver and spleen with blood (mesenteric arteries) can cause abdominal pain after eating and undesired weight loss. FMD in the arms and legs can cause discomfort in the extremities with exercise or may cause uneven blood pressure in the arms. CausesThe cause of FMD is not yet known, but several theories have been suggested. Several case reports in literature have identified the disease in several members of the same family including twins. As a result, it feels that there may be a genetic cause. However, a relative may have a different involvement of the artery, a different severity of the disease, or not developing the FMD at all. In fact, most individuals with FMD do not have a relative who also has the disease. Among some individuals with FMD, there is a family history of other vascular problems, such as blood vessel aneurysms. FMD is much more common in women than in men, which results in the theory that hormones can play an important role in disease development. However, in small population studies, reproductive history (number of pregnancies and when they occurred) as well as the use of birth control pills did not relate to the development of the MDF. Other possible causes of FMD include abnormal development of arteries that supply the wall of the blood vessel, resulting in insufficient supply of oxygen; anatomical position or movement of the artery within the body; certain medicines and tobacco use. Many factors may contribute to the development of FMD. This area requires further research. Affected populations The MDF affects women much more than men, although men and children may be affected by this disease. In children with FMD, the disease seems to be more commonly present with significant narrowing rather than tears of arteries and also seems to involve the arteries to the kidneys and intestines more commonly than the carotic vessels. In the pediatric population, FMD affects both boys and girls. Related disorders The vascular subtype of Ehlers-Danlos syndrome (type IV) has been associated with the most common type of fibromuscular dysplasia, known as multifocal FMD. This syndrome should be suspected in patients with multiple aneurysms and/or tears (dissections) in arteries as well as the typical angiographic findings of fibromuscular dysplasia. Insulated reports of fibromuscular dysplasia associated with other disorders have been received, such as Alport syndrome, pheochromocytoma, Marfan syndrome and moyamoya disease. (For more information on these disorders, search for the term in the rare disease database). Diagnosis For the diagnosis of FMD, a test should be performed for the image of blood vessels. There are many options for the image of the arteries, including the ultrasound of specialized blood vessel known as ultrasound duplex; a CAT scan of the arteries that is obtained after a dye is given through the veins, or a special type of MRI. In many cases, the diagnosis of MDF requires a procedure known as arteriogram. The arteryography is a procedure performed by a radiologist, vascular surgeon, cardiologist or vascular medicine specialist with appropriate training. It is about inserting a wire into or near the affected artery and injecting contrast material, a dye that can be detected by an X-ray machine. An X-ray of the affected area is taken and examined. The individual is usually awake during an arteriogram procedure, although medications can be given to keep it comfortable. This outpatient procedure usually lasts from one to two hours with a recovery period of up to six hours (this varies widely). Standard Therapies Treatment No cure for FMD. Treatments focus on managing symptoms and complications of MDF, including high blood pressure and headaches. Anti-aggregating drugs, such as aspirin, may be prescribed along with medications to treat high blood pressure (anti-hypertensive). Many DFF patients suffer from headaches, and several medications are available to help control and prevent headaches. All DFF patients who use tobacco should be encouraged to leave. In some cases of FMD, you should try to improve the flow of blood through a severely narrow vessel. The type of treatment used to narrow due to disease disorder depends to a large extent on which arteries are affected and on the presence and severity of symptoms. In most cases, these procedures are performed using the balloon angioplasty, a procedure known as percutaneous transluminal angioplasty (PTA). PTA is often performed at the same time as an arteriogram. If an angioplasty is performed, a catheter is extended to the affected artery and a small balloon is inflated in the artery. Normally a metal stent is not required to keep the vessel open, but it may be necessary in some cases, as for the treatment of a tear (dissections) of a blood vessel. If angioplasty is performed, the procedure and the recovery period can be longer than an arteriogram made only for diagnosis. From time to time, traditional open surgery is done to treat severe narrowing due to FMD, particularly those that cannot be treated with angioplasty. Patients with EPF who encounter significant aneurysm within the brain or kidney arteries may need surgery even without symptoms. In such cases, it is recommended that aneurysm be treated to prevent the rupture that may be potentially life threatening. The type of treatment for an arterial aneurysm depends on its location and size. Treatment options for aneurysms include traditional open surgery or a less invasive procedure based on angiograms that treat aneurysm using coils and special vascular stents. The proper treatment will vary with each individual and severity, location and extent of disease. The treatment plan should be discussed in depth with a specialist who is very knowledgeable about the FMD and its natural history. Research The FMD The Society of America is sponsoring the registration of patients from the United States for the FMD, which is gathering information on epidemiology, symptoms, spread of disease, treatments and results of patients with FMD. For more information on the registration of patients with FMD, go to the information on current clinical trials is posted on the Internet. All studies that receive funding from the US government, and some supported by the private industry, are published on this government website. For information on clinical trials being conducted at the National Institutes of Health Clinic (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office: (800) 411-1222 TTY: (866) 411-1010 Email: For information on clinical trials sponsored by private sources, please contact: To get information on clinical trials performed in Europe, contact ResourcesConcerts with rare disease For more information, visit . RareConnect offers a secure online community for patients and caregivers affected by this rare disease. For more information, visit . NORD Member Organizations Other organizationsReferencesTEXTBOOKS Kasper, DL, Fauci AS, Longo DL, et al., eds. Harrison Principles on Internal Medicine.16th ed. McGraw-Hill Companies. New York, NY; 2005:1469-71, 1707-08, 2378.Badr KF, Brenner BM. Vascular injury to the kidney. In: Brenner BM, Rector Jr FC. The Kidney. W.B. Saunders Company. Philadelphia, PA; 1991:1948-49. JOURNAL ARTICLES Green R, Gu X, Kline-Rogers E, Froehlich J, Mace P, Gray B, Katzen B, Olin J, Gornik HL, Cahill AM, Meyers KE. Differences between the pediatric and adult presentation of fibromuscular dysplasia: results of the American registry. Pediatric nephrology. 2015; 1-10. DOI:10.1007/s00467-015-3234-z. Weinberg I, Gu X, Giri J, Kim SE, Bacharach MJ, Gray BH, Katzen BT, Matsumoto AH, Chi YW, Rogers KR, Froehlich J, Olin JW, Gornik HL, Jaff MR. Use of antiplatelet and antihypertension medications in patients with fibromuscular dysplasia: Results of the United States fibromuscular dysplasia registry. Vasc Med. 2015;20(5):447-453. Sharma AM, Kline B. American fibromuscular dysplasia record: new findings and rupture myths. Tech Vasc Interventional Rad. 2014;17(4):258-263. Kim ESH, Olin JW, Froehlich JB, Gu X, Bacharach JM, Gray BH, Jaff MR, Katzen BT, Kline-Rogers E, Mace P, Matsumoto AH, McBane RD, White CJ, Gornik HL. Clinical manifestations of fibromuscular dysplasia vary by patient sex: a report of the United States registry for fibroplasia. JACC. 2013;62(21):2026-2028. Olin JW, Froehlich J, Gu X, Bacharach M, Eagle K, Gray B, Jaff MR, Kim E, Mace P, Matsumoto AH, McBane R, Kline-Rogers E, White C, Gornik HL. The U.S. fibromuscular dysplasia record: results in the first 447 patients. Regulation 2012;125(25):3182-3190. Poloskey SL, Olin JW, Mace P, Gornik HL. Page of patients with fibromuscular dysplasia. Circulation. 2012;125:e636-e639. INTERNET Chen M. Renovascular hypertension. MedlinePlus. Updated 5/5/2016. Available in Login 30 January 2017.McKusick VA, ed. Mendelian heritage online in man (OMIM). Johns Hopkins University. Fibromuscular dysplasia of the arteries. Entry number 135580. Available in Last Edition 09/10/2013. Accessed January 30, 2017. Published Years Information in the NORD rare disease database is only for educational purposes and is not intended to replace the advice of a doctor or other qualified medical practitioner. The content of the website and the databases of the National Organization for Rare Disorders (NORD) is protected by copyright and cannot be reproduced, copied, downloaded or disseminated, in any way, for commercial or public purposes, without prior authorization and written approval of NORD. Individuals may print a printed copy of an individual disease for personal use, provided that the content is not modified and includes NORD copyrights. National Organization for Rare Disorders (NORD) 55 Kenya Ave., Danbury CT 06810 • (203)744-0100 Report Index To search for patient organizations and other pages related to this topic, use the Advanced Search function at the top right corner of the page. Related Content NORD strives to open new assistance programs such as funding allows. If we don't have a program for you now, please continue with us. We're just weird. Together we are strong. ®Copyright ©2021 NORD - National Organization for Rare Disorders, Inc. All rights reserved. NORD is a registered charity organization 501(c)(3). Please note that NORD provides this information for the benefit of the rare disease community. NORD is not a medical provider or a health care center and therefore cannot diagnose any disease or disorder, or endorse or recommend specific medical treatments. Patients should depend on personal and individualized medical advice from their qualified health professionals before seeking information related to their diagnosis, cure or particular treatment of a condition or disorder.

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